Transcript Adult basic life support

Congenital Heart Disease
Faculty of Medicine
University of Brawijaya
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Definition
• Cardiac lesions present from birth
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Causes of congenital heart
disease
• Many factors both genetic and
environmental affect cardiac development
in the uterus; therefore no one cause can
explain all cases
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Causes of congenital heart
disease
• Maternal rubella- in addition to
cataracts, deafness, and microcephaly,
this can cause patent ductus arteriosus
(PDA) and pulmonary stenosis
• Fetal alcohol syndrome- associated with
cardiac defects (as well as
microcephaly, micrognathia,
microphthalmia, and growth retardation)
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Causes of congenital heart
disease
• Maternal systemic lupus erythematosus
– associated with fetal complete heart
block (due to transplacental passage of
anti-Ro antibodies)
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Genetic associations with
congenital heart disease
• Trisomy 21- endocardial cushion defects,
atrial septal defect (ASD), ventricular septal
defect (VSD), tetralogy of Fallot.
• Turner’s syndrome (X0)- coarctation of the
aorta
• Marfan syndrome- aortic dilatation and aortic
and mitral regurgitation
• Kartagener’s syndrome
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Complications of congenital
heart disease
• Cyanosis – the presence of more than
5g/dL of reduced hemoglobin in arterial
blood
• Congestive heart failure – this occurs
due to the inability of the heart to
maintain sufficient tissue perfusion as a
result of the cardiac lesion
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Complications of congenital
heart disease
• Pulmonary hypertension-this occurs as
a result of an abnormal increase in
pulmonary blood flow due to left-to-right
shunt (e.g ASD, VSD, PDA)
• Infective endocarditis – congenital heart
disease may result in lesions prone to
bacterial colonization
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Complications of congenital
heart disease
• Sudden death – this may be due to
arrhythmias (more common in these
disorders) or outflow obstruction as
seen in aortic stenosis
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Classification of cyanotic congenital heart disease
Classification of cyanoctic congenital heart disease by the amount
of pulmonary blood flow seen on chest x ray.
Increased Pulmonary Blood Flow
Normal or Decreased Pulmonary Blood Flow
Tricuspid atresia with large VSD
Tricuspid atresia with restrictive VSD
Total anomalous pulmonary venous return
Pulmonary atresia with intact ventricular septum
Truncus arteriosus
Ebstein’s anomaly
D-transposition of the great arteries
D-transposition of the great arteries with pulmonary
stenosis
Taussig-Bing anomaly
Double outlet right ventricle with pulmonary stenosis
Tetralogy of Fallot with minimal right ventricular
outflow tract obstruction
Tetralogy of Fallot
Tetralogy of Fallot with pulmonary atresia and
increased collateral flow
Tetralogy of Fallot wit pulmonary atresia
Single ventricle without pulmonary stenosis
Single ventricle with pulmonary stenosis
Interrupted aortic arch with PDA
Vena cava to left atrium communication
Hypoplastic left heart syndrome
ASD with Eisenmenger’s syndrome
VSD with Eisenmenger’s syndrome
PDA with Eisenmenger’s syndrome
Acyanotic heart disease
Chest X
ray
Blood flow to lung
Normal
RVH
-PS
-MS
Increased
LVH
-MI
-AS
-KoA
RVH
-ASD
-PAPVR
-PVOD
LVH/BVH
-VSD
-PDA
-AVSD
Echocardiography, cardiac catheterization
EKG
Cardiac malformations
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Ventricular septal defect (VSD)
Atrial septal defect (ASD)
Patent ductus arteriosus (PDA)
Pulmonary stenosis – causes cyanosis if severe
Coarctation of the aorta
Aortic stenosis
Tetralogy of Fallot – causes cyanosis
Transposition of the great arteries – causes cyanosis
Other causes of cyanotic congenital heart disease-pulmonary
atresia, hypoplastic left heart, severe Ebstein’s anomaly with
ASD
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Patofisiologi left to right shunt
Of left to right shunt:
 volume burden
 dilatation/hipertrophy
ASD
VSD
PDA
Atrial Septal Defect (ASD)
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3 types of Atrial septal defect
• (1) Septum primum (ostium primum
ASD)- this defect lies adjacent to
atrioventricular valves, which are often
also abnormal and incompetent
• (2) Septum secundum (ostium
secundum ASD)- the most common
form of ASD, it is midseptal in location
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3 types of Atrial septal defect
• (3) Sinus venosus ASD – this lies high
in the septum and may be associated
with anomalous pulmonary venous
drainage (in which one of the pulmonary
veins drains into the right atrium instead
of the left.
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Clinical features
• The magnitude of the left-to-right shunt
depends upon the size of the defect and
also the relative pressures on the left
and right sides of the heart.
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History
• Early life: asymptomatic
• Adult life: dyspnea, fatigue, recurrent
chest infections
• As time goes by, the increased
pulmonary blood flow results in
pulmonary hypertension and eventually
reversal of the shunt and Eisenmenger
syndrome
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Examination
• The findings of a patient who has an
ASD depend upon the following factors:
– Size of ASD
– Presence or absence of pulmonary
hypertension
– Present of reversal
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Examination
• The second heart sound is widely split
because closure of the pulmonary valve is
delayed due to increased pulmonary blood
flow.
• The splitting is fixed in relation to respiration
because the communication between the
atria prevents the normal pressure differential
between right and left sides that occurs
during respiration.
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Examination
• The increased pulmonary blood flow causes
a mid systolic pulmonary flow murmur.
• If PH has developed  reduction of the leftto-right shunt, the pulmonary flow murmur
disappears; there is a loud pulmonary
component to the second heart sound
• If Eisenmenger’s syndrome occurs 
centrally cyanosed, finger clubbing
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Investigations
• EKG
– Ostium secundum ASD: right axis deviation
– Ostium primum defect: left axis deviation
• Chest radiography
– Pulmonary arteries: dilated, its branches are
prominent
– Enlarged right atrium, enlarged right ventricle
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Echocardiography
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Echocardiography
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Cardiac catheterization
• To reveal ASD, because the catheter can be
passed across it.
• Serial oxygen saturation measurements are
made at different levels from the superior
vena through the atrium and the right
ventricle into the pulmonary artery.
• At the level of the left-to-right shunt there will
be a step up increase of the oxygen
saturation as blod flow from the left side
enters the right.
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Management of ASD
• Signs of congestive heart failure:
diuretics and ACE inhibitors
• ASD carries a risk of infective
endocarditis  appropriate prophylactic
measures should be taken
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Management of ASD
• Early diagnosis  evaluate its severity
to be able to repair the defect before
pulmonary hypertension occurs.
• If PH (+), repair does not stop its
deterioration. ASD with pulmonary to
systemic flow ratio exceeding 1,5:1
should be repaired.
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Ventricular Septal Defect
(VSD)
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VSD
• The most common congenital cardiac
abnormality
• The ventricular septum is made up of
two main components:
– The membraneous septum- situated high
in the septum and relatively small. The
most common site for a VSD.
– The muscular septum- lower and defects
here may be multiple.
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Clinical features
• Neonates:
– Small VSD: asymptomatic
– Large VSD  left ventricular failure :
• Failure to thrive, feeding difficulty, sweating on
feeding
• Tachypnea and intercostal recession
• Hepatomegaly
• Adult:
– Asymptomatic
– Dyspnea due to PH or Eisenmenger syndrome
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Examination
Findings depend on:
• Size of VSD:
• small VSD: loud holosystolic murmur, radiates to
the apex and axilla
• Loud VSD: less loud holosystolic murmur with
signs of left and right ventricular hypertrophy
• Presence or absence of pulmonary
hypertension
• Presence of shunt reversal
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Investigations
• Chest radiography: enlarged left
ventricle with prominent pulmonary
vascular markings. Pulmonary edema
may be seen in infants.
• Echocardiography: shows VSD and its
size and location
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Echocardiography
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Echocardiography
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Management
• 30% of cases close spontaneously,
mostly by the time the child is 3 years of
age.
• Some do not close until the child is 10
years old.
• Defects near the valve ring or near the
outlet of the ventricle do not usually
close
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Management
• Operative closure is the treatment of
choice and is recommended for all
lesions that have not undergone
spontaneous closure
• VSD  risk factor for infective
endocarditis  appropriate prophyalctic
measures should be taken
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Patent ductus arteriosus
(PDA)
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PDA
• In the fetus most of the output of the right
ventricle bypasses the lungs via the ductus
arteriosus
• This vessel joins the pulmonary trunk (artery)
to the descending aorta distal to the left
subclavian artery.
• The ductus arteriosus normally closes about
1 month after birth in full-term infants and
takes longer to close in premature infants
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Clinical features
• The factors that determine the nature of
clinical features are the same as in VSD and
ASD i.e the size of the defect, the presence of
PH, the development of Eisenmenger’s
syndrome
• A patent PDA is more likely in babies born at
high altitude, probably due to low
atmospheric oxygen concentration; it may
also occur In babies who have fetal rubella
syndrome
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History
• Small PDA: asymptomatic
• Large PDA: large left-to-right shunt 
left ventricular failure with pulmonary
edema causing failure to thrive and
tachypnea
• Adults with undiagnosed PDA may
develop PH and present with dyspnea
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Examination
• Collapsing high-volume pulses – this is
due to the effect of the run-off of blood
back down the ductus
• Loud continuous machinery murmur
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Management
• Pharmacological closure in neonates –
indomethacin may induce closure if
given early
• Operative closure – this can be
performed as an open procedure in
which the PDA is ligated or divided
• Or using ADO (Amplatzer ductal
occluder) by cardiac catheter
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Management
• PDA is a risk fractor for infective
endocarditis  antibiotic prophylaxis is
required for all patients before operative
procedures.
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Eisenmenger’s syndrome
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Eisenmenger’s syndrome
• Refers to the situation in which a
congenital cardiac abnormality initially
causes acyanotic heart disease, but
cyanotic heart disease develops as a
consequence of raised pulmonary
pressure and shunt reversal
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Eisenmenger’s syndrome
• These clinical features are also seen in
patients who have cyanotic congenital
heart disease
• Cyanosis develops when the level of
reduced hemoglobin is over 5 g/dL.
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Complications of
Eisenmenger’s syndrome
• Clubbing fingers and toes
• Polycythemia and hyperviscosity- with
resulting complications of stroke and venous
thrombosis. Regular phlebotomy is the
treatment of choice
• Cerebral abscesses-especially in children
• Paradoxical emboli- emboli from venous
thrombosis may pass across the shunt and
give rise to systemic infarcts
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Clubbing fingers and toes
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